Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome

Pumin Zhang, Nanette J. Liégeois, Calvin Wong, Milton Finegold, Harry Hou, Janet C. Thompson, Adam Silverman, J. Wade Harper, Ronald A. DePinho, Stephen J. Elledge

Research output: Contribution to journalArticle

596 Citations (Scopus)

Abstract

Mice lacking the imprinted Cdk inhibitor p57(KIP2) have altered cell proliferation and differentiation, leading to abdominal muscle defects; cleft palate; endochondral bone ossification defects with incomplete differentiation of hypertrophic chondrocytes; renal modullary dysplasia; adrenal cortical hyperplasia and cytomegaly; and lens cell hyperproliferation and apoptosis. Many of these phenotypes are also seen in patients with Beckwith-Wiedemann syndrome, a pleiotropic hereditary disorder characterized by overgrowth and predisposition to cancer, suggesting that loss of p57(KIP2) expression may play a role in the condition.

Original languageEnglish (US)
Pages (from-to)151-158
Number of pages8
JournalNature
Volume387
Issue number6629
DOIs
StatePublished - May 8 1997

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Beckwith-Wiedemann Syndrome
Abdominal Muscles
Cleft Palate
Chondrocytes
Osteogenesis
Lenses
Hyperplasia
Cell Differentiation
Cell Proliferation
Apoptosis
Phenotype
Kidney
Bone and Bones
Neoplasms

ASJC Scopus subject areas

  • General

Cite this

Zhang, P., Liégeois, N. J., Wong, C., Finegold, M., Hou, H., Thompson, J. C., ... Elledge, S. J. (1997). Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome. Nature, 387(6629), 151-158. https://doi.org/10.1038/387151a0

Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome. / Zhang, Pumin; Liégeois, Nanette J.; Wong, Calvin; Finegold, Milton; Hou, Harry; Thompson, Janet C.; Silverman, Adam; Harper, J. Wade; DePinho, Ronald A.; Elledge, Stephen J.

In: Nature, Vol. 387, No. 6629, 08.05.1997, p. 151-158.

Research output: Contribution to journalArticle

Zhang, P, Liégeois, NJ, Wong, C, Finegold, M, Hou, H, Thompson, JC, Silverman, A, Harper, JW, DePinho, RA & Elledge, SJ 1997, 'Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome', Nature, vol. 387, no. 6629, pp. 151-158. https://doi.org/10.1038/387151a0
Zhang, Pumin ; Liégeois, Nanette J. ; Wong, Calvin ; Finegold, Milton ; Hou, Harry ; Thompson, Janet C. ; Silverman, Adam ; Harper, J. Wade ; DePinho, Ronald A. ; Elledge, Stephen J. / Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome. In: Nature. 1997 ; Vol. 387, No. 6629. pp. 151-158.
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